Cystic fibrosis: a cell culture study on an adult patient population.

Skin fibroblast cultures from an adult population of cystic fibrosis (CF) patients 
have been studied to determine any possible genetic or clinical significance 
of the two classes described for cultured CF skin fibroblasts (Danes 
1973). On the basis of clinical course, the 46 adult patients 
from 43 unrelated families studied were divided into two groups (40 
typical, 6 atypical). The cultured fibroblasts from 37 of the 40 
patients with typical CF were Class I (metachromatic, cystic fibrosis factor 
activity (CFFA) in the culture medium, and metabolic cooperation with normal 
fibroblasts), and the cultures from the remaining three patients were Class 
II (ametachromatic, no CFFA in the culture medium and no metabolic 
cooperation with metachromatic CF fibroblasts). During the 4-year research period one 
of the 37 patients from Class I and all three Class 
II patients with typical CF succumbed. The cultured skin fibroblasts from 
two of the six patients who had an atypical course for 
CF were Class I and four were Class II. When the 
parents of three of these atypical CF Class II patients were 
studied, the cultured fibroblasts from one parent in each family in 
each family were Class I and the other Class II. The 
affected offspring from such Class I-II matings may represent genetic compounds. 
